Link, Michael J.

Abstract Pediatric craniopharyngioma is a rare sellar-region epithelial tumor that, in spite of its typically benign pathology, has the potential to be clinically devastating, and presents a host of formidable management challenges for the skull base surgeon. Strategies in craniopharyngioma care have been the cause of considerable controversy, with respect to both philosophical and technical issues. Key questions remain unresolved, and include optimizing extent-of-resection goals; the ideal radiation modality and its role as an alternative, adjuvant, or salvage treatment; appropriate indications for expanded endoscopic endonasal surgery as an alternative to transcranial microsurgery; risks and benefits of skull base techniques in a pediatric population; benefits of and indications for intracavitary therapies; and the preferred management of common treatment complications. Correspondingly, we sought to review the preceding basic science and clinical outcomes literature on pediatric craniopharyngioma, so as to synthesize overarching recommendations, highlight major points of evidence and their conflicts, and assemble a general algorithm for skull base surgeons to use in tailoring treatment plans to the individual patient, tumor, and clinical course. In general terms, we concluded that safe, maximal, hypothalamic-sparing resection provides very good tumor control while minimizing severe deficits. Endoscopic endonasal, intraventricular, and transcranial skull base technique all have clear roles in the armamentarium, alongside standard craniotomies; these roles frequently overlap, and may be further optimized by using the approaches in adaptive combinations. Where aggressive subtotal resection is achieved, patients should be closely followed, with radiation initiated at the time of progression or recurrence—ideally via proton beam therapy, although three-dimensional conformal radiotherapy, intensity-modulated radiotherapy, and stereotactic radiosurgery are very appropriate in a range of circumstances, governed by access, patient age, disease architecture, and character of the recurrence. Perhaps most importantly, outcomes appear to be optimized by consolidated, multidisciplinary care. As such, we recommend treatment in highly experienced centers wherever possible, and emphasize the importance of longitudinal follow-up—particularly given the high incidence of recurrences and complications in a benign disease that effects a young patient population at risk of severe morbidity from hypothalamic or pituitary injury in childhood.

Abstract Cancer patient survivorship has become a significant topic within oncology care for both adult and pediatric patients. Starting in 2005, the Institute of Medicine recommended the use of survivorship care plans to assist patients transitioning from active treatment to the posttreatment phase of their cancer care, a critical time for many patients. Since 2014 there has been a mandate within the United States for adult cancer patients treated with curative intent to receive survivorship care plans comprised of a treatment summary and a follow-up plan to facilitate a better understanding among patients of what to expect after treatment. In addition to a general oncology survivorship care plan, specific care plans have been created for breast, lung, prostate, and colon cancers, as well as lymphoma. A survivorship care plan specific to adult neuro-oncology has been developed by a multidisciplinary and interprofessional committee, with approval from the Society for Neuro-Oncology Guidelines Committee. It has been published in compendium with this review of survivorship care planning and available as a fillable PDF on the Society of Neuro-Oncology Guidelines Endorsement web page (https://www.soc-neuro-onc.org/SNO/Resources/Survivorship_Care_Plan.aspx). Implementation of this survivorship care plan provides a unique opportunity to begin addressing the range of survivorship issues our neuro-oncology patients navigate from diagnosis to end of life.

Abstract BACKGROUND Stereotactic radiosurgery (SRS) is a commonly performed procedure for patients with intracranial meningiomas. OBJECTIVE To describe the clinical features of patients with radiation-induced cavernous malformations (RICM) after single-fraction meningioma SRS. METHODS Retrospective study of patients having single-fraction SRS for intracranial meningioma at our center from 1990 through 2009, and 1 patient who had single-fraction SRS elsewhere. Patients were excluded if they refused research authorization (n = 7), had a World Health Organization Grade II or III meningioma (n = 65), had a genetic predisposition for tumor development (n = 52), had prior or concurrent radiation therapy (n = 49), or had less than 2 yr of magnetic resonance imaging follow-up after SRS (n = 77). The median follow-up of the remaining 426 patients was 7.9 yr (range, 2-24.9). RESULTS Three RICM (0.7%) were identified at 2, 10, and 21 yr after SRS. Two patients were asymptomatic, whereas 1 patient had a brainstem hemorrhage causing facial weakness and numbness. The risk of developing an RICM after SRS was 0.2% at 5 yr and 0.9% at 15 yr. All patients were observed and remained stable without additional bleeding in follow-up of 7, 12.8, and 2 yr, respectively. A fourth patient developed progressive neurological dysfunction starting 7 yr after SRS at another center and was treated for several years with bevacizumab without improvement. Surgical resection was performed 11.5 yr after SRS and histologic examination was consistent with an RICM. CONCLUSION The risk of RICM after single-fraction SRS for intracranial meningiomas is very low, but the latency period noted until their detection emphasizes the need for extended imaging follow-up after SRS of benign lesions.

Objective: To describe audiometric outcomes following stereotactic radiosurgery (SRS) for jugular paraganglioma (JP). Study Design: Retrospective review. Setting: Tertiary referral center. Patients: Patients with pretreatment serviceable hearing (American Academy of Otolaryngology—Head and Neck Surgery [AAO-HNS] Class A or B) and serial post-SRS audiometric follow-up who underwent Gamma Knife SRS for JP between 1990 and 2017. Intervention(s): Gamma Knife SRS. Main Outcome Measures: Progression to nonserviceable hearing; correlation between baseline hearing and treatment parameters with audiometric outcomes. Results: Of 85 patients with JP who underwent SRS during the study period, 35 (66% female, median age 53) had pretreatment serviceable hearing and serial post-treatment audiometry available for review. Median tumor volume at the time of treatment was 7,080 mm3, median cochlear point dose was 5.8 Gy (interquartile range [IQR] 4.1 to 7.3 Gy), and median marginal and maximum tumor doses were 16 and 32 Gy, respectively. After a median follow-up of 37 months (IQR 16 to 77 mo), the median change in pure-tone average and speech discrimination score in the treated ear was −1.2 dB HL/yr (IQR −4.5 to 0.3) and 0%/yr (IQR 0–3.5%), compared with 0.07 dB HL/yr (IQR −0.03 to 0.12) and 0 %/yr (IQR 0 to 0%) in the contralateral untreated ear. Seven patients developed nonserviceable hearing (AAO-HNS Class C or D) at a median of 13.2 months following SRS (IQR 4.8 to 24 mo). Among those who maintained serviceable hearing, median audiometric follow-up was 42 months (IQR 18 to 77 mo). The Kaplan–Meier estimated rates of serviceable hearing at 1, 3, and 5 years following SRS were 91%, 80%, and 80%, respectively. Sixty percent of patients with pulsatile tinnitus who underwent SRS experienced varying levels of symptomatic improvement following treatment. Conclusion: The short- and intermediate-term risk of progression to nonserviceable hearing following SRS for JP is low. Data regarding the impact of cochlear dose from the vestibular schwannoma literature should not be freely applied to JP, since the impact of SRS parameters on hearing preservation seems to be less significant.

Background Very few studies have examined vestibular schwannoma (VS) management trends across centers and between providers. The objective of this study is to examine current practice trends, variance in treatment philosophies, and nuanced or controversial aspects of VS care across North America. Methods This is a cross-sectional survey of North American Skull Base Society (NASBS) members who report regular involvement in VS care. Results A total of 57 completed surveys were returned. Most respondents claimed to have over 20 years of experience and the majority reported working in an academic practice with an affiliated otolaryngology and/or neurosurgery residency program. Sixty-three percent of respondents claimed to evaluate VS patients in clinic with both an otolaryngologist and neurosurgeon involved. Eighty-six percent of respondents claimed to operate on VS with both an otolaryngologist and neurosurgeon involved, while only 18% of neurosurgeons and 9% of otolaryngologists performed surgery alone. There was a wide range in the number of cases evaluated at each center annually. Similarly, there was wide variation in the number of patients treated with microsurgery and radiation at each center. Additional details regarding management preferences for microsurgery, stereotactic radiosurgery, stereotactic radiotherapy, and conservative observation are presented. Conclusion VS management practices vary between providers and centers. Overall, most centers employ a multidisciplinary approach to management with collaboration between otolaryngology and neurosurgery. Overall, survey responses concur with previous studies suggesting a shift toward conservatism in management.

Introduction Perioperative care of vestibular schwannoma (VS) patients is extremely variable across surgeons and institutions making practice patterns difficult to standardize. No data currently exist detailing this practice variability. Methods The North American Skull Base Society membership was electronically surveyed regarding perioperative care of surgically operated VS patients. Results There were 87 respondents to the survey. Surgical positioning, surgical approach utilized, and perioperative medical adjuncts are quite variable. However, of those performing retrosigmoid approaches, 49% perform this in the supine position, while 33% use a park-bench position with only 2% using the sitting position. In those performing translabyrinthine approaches, 86% perform this in supine position. Although the use of neuromonitoring appears to be standard of care (98%), other than the seventh nerve, there is substantial variability between respondents regarding monitoring of additional cranial nerves. Postoperative antibiotics are used by 65%, postoperative steroids 81%, and postoperative chemical deep vein thrombosis prophylaxis in 68% of survey respondents. Conclusion Although the perioperative adjuncts to VS surgery are variable, there does appear to be a trend in common practice. Therefore, making surgeons aware of these trends may lead to standardized practice or alternatively trials of these variances to instruct which truly improve patient outcomes.

Abstract Please see the full-text version of this guideline (https://www.cns.org/guidelines/guidelines-management-patients-vestibular-schwannoma/chapter_8) for the target population of each recommendation listed below. QUESTION 1 What surgical approaches for vestibular schwannomas (VS) are best for complete resection and facial nerve (FN) preservation when serviceable hearing is present? RECOMMENDATION There is insufficient evidence to support the superiority of either the middle fossa (MF) or the retrosigmoid (RS) approach for complete VS resection and FN preservation when serviceable hearing is present. QUESTION 2 Which surgical approach (RS or translabyrinthine [TL]) for VS is best for complete resection and FN preservation when serviceable hearing is not present? RECOMMENDATION There is insufficient evidence to support the superiority of either the RS or the TL approach for complete VS resection and FN preservation when serviceable hearing is not present. QUESTION 3 Does VS size matter for facial and vestibulocochlear nerve preservation with surgical resection? RECOMMENDATION Level 3: Patients with larger VS tumor size should be counseled about the greater than average risk of loss of serviceable hearing. QUESTION 4 Should small intracanalicular tumors (<1.5 cm) be surgically resected? RECOMMENDATION There are insufficient data to support a firm recommendation that surgery be the primary treatment for this subclass of VSs. QUESTION 5 Is hearing preservation routinely possible with VS surgical resection when serviceable hearing is present? RECOMMENDATION Level 3: Hearing preservation surgery via the MF or the RS approach may be attempted in patients with small tumor size (<1.5 cm) and good preoperative hearing. QUESTION 6 When should surgical resection be the initial treatment in patients with neurofibromatosis type 2 (NF2)? RECOMMENDATION There is insufficient evidence that surgical resection should be the initial treatment in patients with NF2. QUESTION 7 Does a multidisciplinary team, consisting of neurosurgery and neurotology, provides the best outcomes of complete resection and facial/vestibulocochlear nerve preservation for patients undergoing resection of VSs? RECOMMENDATION There is insufficient evidence to support stating that a multidisciplinary team, usually consisting of a neurosurgeon and a neurotologist, provides superior outcomes compared to either subspecialist working alone. QUESTION 8 Does a subtotal surgical resection of a VS followed by stereotactic radiosurgery (SRS) to the residual tumor provide comparable hearing and FN preservation to patients who undergo a complete surgical resection? RECOMMENDATION There is insufficient evidence to support subtotal resection (STR) followed by SRS provides comparable hearing and FN preservation to patients who undergo a complete surgical resection. QUESTION 9 Does surgical resection of VS treat preoperative balance problems more effectively than SRS? RECOMMENDATION There is insufficient evidence to support either surgical resection or SRS for treatment of preoperative balance problems. QUESTION 10 Does surgical resection of VS treat preoperative trigeminal neuralgia more effectively than SRS? RECOMMENDATION Level 3: Surgical resection of VSs may be used to better relieve symptoms of trigeminal neuralgia than SRS. QUESTION 11 Is surgical resection of VSs more difficult (associated with higher facial neuropathies and STR rates) after initial treatment with SRS? RECOMMENDATION Level 3: If microsurgical resection is necessary after SRS, it is recommended that patients be counseled that there is an increased likelihood of a STR and decreased FN function. The full guideline can be found at: https://www.cns.org/guidelines/guidelines-management-patients-vestibular-schwannoma/chapter_8.

Abstract BACKGROUND When clinical presentation, laboratory studies, or imaging cannot diagnose cavernous sinus (CS) and/or Meckel's cave (MC) lesions, biopsy may be necessary. OBJECTIVE To review our institutional series of biopsies of indeterminate CS and MC lesions. METHODS Records from January 1994 to June 2016 were searched for biopsied indeterminate CS and MC lesions. We defined indeterminate as having an atypical imaging appearance or a broad differential and the need for tissue for definitive diagnosis. We defined primary tumors as originating from cells inherent or near the CS and MC. RESULTS Eighty-five patients were included (median age 59 [2-85] yr); 22 (28%) had a cancer history. Approaches included frontotemporal craniotomy (n = 48, 56%), endoscopic endonasal (n = 20, 24%), percutaneous transforamen ovale (n = 12, 14%), or retrosigmoid craniotomy (n = 5, 6%). Final diagnosis was metastatic in 27 (32%), primary in 21 (25%), inflammatory in 13 (15%), hematologic in 11 (13%), fungal in 5 (5%), and nondefinitive or nondiagnostic in 8 (10%) patients. Thirteen (59%) patients with a cancer history (n = 22) had a diagnosis consistent with their prior cancer; the remaining had a second pathology (n = 6, 27%) or nondiagnostic biopsy (n = 3, 14%). Two patients had surgical complications resulting in death. CONCLUSION In this patient cohort, metastatic tumors were the most likely pathology. The biopsy threshold should be lower in patients with a cancer history if clinical or radiographic diagnosis is uncertain as 27% had a second disease. However, we consider biopsy as a last resort because the risk of major morbidity/mortality, while low, is not zero.

Objective Olfactory preservation after resection of esthesioneuroblastoma (ENB) has been reported, however, the ability to predict tumor involvement of the olfactory system is critical to this surgical strategy. This study aims to answer the question: Can a surgeon predict, based on preoperative imaging, whether there is unilateral involvement of the olfactory system allowing for safe attempt of olfactory preservation? Methods This is a retrospective review of post-resection ENB meeting inclusion criteria of having bilateral olfactory tracts and bulbs submitted at the time of primary resection for pathologic margins. Five board-certified skull base surgeons blinded to the pathology individually reviewed the preoperative MRI scans to predict degree of tumor involvement. Results Olfactory bulb involvement occurred in both bulbs in 35% of cases and unilateral in 39% of cases, and there was no involvement in 26% of cases sampled. When comparing physician prediction of involved tracts or bulbs, involvement was appropriate or over-called (i.e., called positive when pathology was in fact negative) in 96% of cases. Conclusion This study demonstrates unilateral or no pathologic olfactory involvement of the olfactory system in 65% of cases. Our ability to predict this involvement, which may allow for a management strategy that attempts to preserve olfactory function, was accurate at 96%. Therefore, interpretation of imaging and proceeding with smell preservation in ENB appears reasonable in this cohort.Level of Evidence: Level 2b.